BAG3 Protein Is Over-Expressed in Endometrioid Endometrial Adenocarcinomas.

Endometrioid endometrial cancer is the most common gynaecological tumor in developed countries, and its incidence is increasing. The definition of subtypes, based on clinical and endocrine features or on histopathological characteristics, correlate to some extent with patient's prognosis, but there is substantial heterogeneity within tumor types. The search for molecules and mechanisms implied in determining the progression and the response to therapy for this cancer is still ongoing. BAG3 protein, a member of BAG family of co-chaperones, has a pro-survival role in several tumor types. BAG3 anti-apoptotic properties rely on its characteristic to bind several intracellular partners, thereby, modulating crucial events such as apoptosis, differentiation, cell motility, and autophagy. BAG3 expression in human endometrial cancer tissues was not investigated so far. Here, we show that BAG3 protein levels are elevated in tumoral and hyperplastic cells in respect to normal glands. Furthermore, BAG3 subcellular localization appears to be changed in tumoral compared to normal cells. Our results indicate a possible role for BAG3 protein in the maintenance of cell survival in endometrioid endometrial cancer and suggest that this field of studies is worthy of further investigations. J. Cell. Physiol. 232: 309-311, 2017. © 2016 Wiley Periodicals, Inc.

Ultrasound-guided fine needle aspiration cytology of a primary lymph node leiomyoma: a flexible procedure for a complex case.

BACKGROUND: A primary lymph node leiomyoma diagnosed by fine needle aspiration cytology (FNAC) is reported. CASE: A 22-year-old male complained of right groin swelling; ultrasound examination (US) showed a lymph node containing a 20-mm hypoechoic nodule. The residual lymph node was oval, with a well-characterized cortex and hilum. US-FNAC of the nodule showed oval spindle cells embedded in fibrillar matrix. Nuclei were naked and oval with dispersed chromatin but without nucleoli. Immunocytochemistry showed positivity for vimentin and actin, and negativity for cytokeratin, S100, CD23 and CD31. A smear of the residual lymph node showed a reactive lymphoid cell population. FNAC diagnosis was mesenchymal cell proliferation with smooth muscle phenotype; a lymph node is part of the lesion. A CT scan did not detect any inguinal or abdominal mass. The surgical sample was a lymph node containing a spindle cell tumor, which was actin and desmin positive, and S100, CD21, HMB45, CD23 and CD31 negative; MIB1 was positive in <5% of the cells. The residual lymph node was normal. CONCLUSION: The final diagnosis was primary benign leiomyoma in a lymph node. US-FNAC may frame complex lymph node lesions and provide treatment options.

Kaposi's sarcoma associated with chronic myeloid leukemia and imatinib mesylate therapy.

Kaposi's sarcoma is associated with immunosuppression and human herpesvirus 8 infection, while rarely described in myeloid malignancies. Here, we illustrate a rare case of chronic myeloid leukemia treated with imatinib, a tyrosine kinase inhibitor, who developed a human herpesvirus 8-related Kaposi's sarcoma.

Hodgkin Lymphoma and Hairy Cell Leukemia Arising from Chronic Lymphocytic Leukemia: Case Reports and Literature Review.

Richter's syndrome represents the progression of chronic lymphocytic leukemia (CLL) to more aggressive diseases, most frequently diffuse large B-cell lymphoma, while Hodgkin's lymphoma (HL) and hairy cell leukemia (HCL) are rarely described. The first case involved a 67-year-old man with a diagnosis of a high-risk stage-II CLL treated with rituximab and ibrutinib, developed a HL nodular sclerosis variant after three months of therapy for CLL. After achieving a complete remission for HL and ibrutinib cessation because of drug-related cardiotoxicity, the patient relapsed after five months off-therapy and died due to disease progression after two cycles of brentuximab-vedotin. The second case involved an 83-year-old female with a diagnosis of stage-IV CLL treated with rituximab plus bendamustine who developed a HCL eight years later. Pentostatin was unsuccessfully employed as upfront HCL therapy, and the patient was then switched to rituximab while in remission for CLL. In conclusion, Richter's transformation risk rate might be higher in patients treated with novel targeted therapies, and multiparametric flow cytometry and lymph node biopsy at relapse could help in early identifying small clones. The treatment of predominant neoplasia is mandatory, and disease-specific drugs are administered; however, clinical efficacy might be lower in these patients.

Hepatic Presentation of Late-Onset Multiple Acyl-CoA Dehydrogenase Deficiency (MADD): Case Report and Systematic Review.

Diagnosis of pediatric steatohepatitis is a challenging issue due to a vast number of established and novel causes. Here, we report a child with Multiple Acyl-CoA Dehydrogenase Deficiency (MADD) presenting with an underrated muscle weakness, exercise intolerance and an atypically severe steatotic liver involvement. A systematic literature review of liver involvement in MADD was performed as well. Our patient is a 11-year-old otherwise healthy, non-obese, male child admitted for some weakness/asthenia, vomiting and recurrent severe hypertransaminasemia (aspartate and alanine aminotransferases up to ×20 times upper limit of normal). Hepatic ultrasound showed a bright liver. MRI detected mild lipid storage of thighs muscles. A liver biopsy showed a micro-macrovacuolar steatohepatitis with minimal fibrosis. Main causes of hypertransaminasemia were ruled out. Serum aminoacids (increased proline), acylcarnitines (increased C4-C18) and a large excretion of urinary glutaric acid, ethylmalonic, butyric, isobutyric, 2-methyl-butyric and isovaleric acids suggested a diagnosis of MADD. Serum acylcarnitines and urinary organic acids fluctuated overtime paralleling serum transaminases during periods of illness/catabolic stress, confirming their recurrent nature. Genetic testing confirmed the diagnosis [homozygous c.1658A > G (p.Tyr553Cys) in exon 12 of the ETFDH gene]. Lipid-restricted diet and riboflavin treatment rapidly ameliorated symptoms, hepatic ultrasonography/enzymes, and metabolic profiles. Literature review (37 retrieved eligible studies, 283 patients) showed that liver is an extramuscular organ rarely involved in late-onset MADD (70 patients), and that amongst 45 patients who had fatty liver only nine had severe presentation. Conclusion: MADD is a disorder with a clinically heterogeneous phenotype. Our study suggests that MADD warrants consideration in the work-up of obesity-unrelated severe steatohepatitis.

Does placement of a connective tissue graft improve the outcomes of coronally advanced flap for coverage of single gingival recessions in upper anterior teeth? A multi-centre, randomized, double-blind, clinical trial.

AIMS: This parallel-group, multi-centre, double-blind, randomized-controlled clinical trial was undertaken to compare the clinical outcomes and patient morbidity of coronally advanced flap (CAF) alone or in combination with a connective tissue graft (CAF+CTG) in single Miller Class I and II gingival recessions. MATERIAL AND METHODS: Three centres enrolled 85 patients with one recession each. Surgery was performed elevating a pedicle flap; 42 sites randomly received a graft under the flap. Measurements were taken by blind and calibrated examiners. Outcome measures included recession reduction, complete root coverage (CRC), intra-operative and post-operative morbidity, dentine sensitivity, and side effects. RESULTS: No differences were noted in the intra-operative and post-operative patient-related variables between the two groups. Surgical time was significantly shorter in the CAF group. Recession reduction was not statistically different between the two groups, even though a model showed a tendency towards improved outcomes in sites treated with CAF+CTG (adjusted difference 0.33 mm, 95% CI=-0.06 to 0.72, p=0.1002). Significantly greater probability of CRC was observed after CAF+CTG (adjusted OR=5.09, 95% CI=1.69-17.57, p=0.0033). Dentine hypersensitivity improved in both the groups. CONCLUSIONS: Both treatments were effective in providing a significant reduction of the baseline recession and dentine hypersensitivity, with only limited intra-operative and post-operative morbidity and side effects. Adjunctive application of a CTG under a CAF increased the probability of achieving CRC in maxillary Miller Class I and II defects.

A rare case of squamous cell carcinoma of a proliferating trichilemmal tumour.

INTRODUCTION: Squamous Cell Carcinoma (SCC) includes several subtypes and these can be classified according to their clinical behaviours from the least invasive to the most aggressive. Moreover, it is appropriate to analyse their clinicalpathological patterns 1. In light of its wide histopathological variability, we encountered a rare malignant cutaneous lesion: a SCC of a proliferating trichilemmal tumour (PTT) nature. CASE REPORT: We describe the clinical and pathologic findings of an 82-year-old woman, who developed a SCC with areas of trichilemmal differentiation. It has a surprising thickness of 25 mm, which passes through the dermis, infiltrates the hypodermis and evolves quickly. CONCLUSION: Considering its exophytic growth and the negative results of the post operative tests (ultrasounds confirmed absence of metastasis), the tumour was eradicated thanks to the surgical approach (a 1 cm border wide excision, including the periosteum in the central part of the lesion, followed by its reconstruction using a full-thickness skin graft) and the prompt intervention, without need for further complimentary treatments. The overall aesthetic results were pleasing. KEY WORDS: Adnexal tumour, PPT, SCC, Surgery.

The first case of benign multicystic mesothelioma presenting as a splenic mass.

Multicystic mesothelioma (MM) is a relatively rare tumour arising in the pelvic peritoneum of the tuboovarian region of young woman. Exceptionally, MM occurs on the serosal surfaces of various organs including kidney, bladder, lymph nodes, and liver. We report here the first case of MM wherein a 58-year-old woman with a previous history of endometriosis of the right ovary presented with a large multicystic mass of the spleen. The diagnosis of MM was made on a surgical specimen after splenectomy. A histopathologic examination is always necessary for the diagnosis of MM which should be differentiated from other lesions particularly from cystic lymphangioma. At one year follow-up, the patient had no evidence of recurrence. Despite the high frequency of local recurrences, MM is a benign lesion and 'en bloc' surgical excision with prolonged follow-ups is the treatment of choice.

CD10, BCL6, and MUM1 expression in diffuse large B-cell lymphoma on FNA samples.

BACKGROUND: Gene expression profiling has divided diffuse large B-cell lymphoma (DLBCL) into 2 main subgroups: germinal center B (GCB) and non-GCB type. This classification is reproducible by immunohistochemistry using specific antibodies such as CD10, B-cell lymphoma 6 (BCL6), and multiple myeloma oncogene 1 (MUM1). Fine-needle aspiration (FNA) plays an important role in the diagnosis of non-Hodgkin lymphoma, and in some cases FNA may be the only available pathological specimen. The objectives of the current study were to evaluate CD10, BCL6, and MUM1 immunostaining on FNA samples by testing the CD10, BCL6, and MUM1 algorithm on both FNA cell blocks (CB) and conventional smears (CS), evaluating differences in CB and CS immunocytochemical (ICC) performance, and comparing results with histological data. METHODS: Thirty-eight consecutive DLBCL cases diagnosed by FNA were studied. Additional passes were used to prepare CB in 22 cases and CS in 16 cases; the corresponding sections and smears were immunostained using CD10, BCL6, and MUM1 in all cases. The data obtained were compared with histological immunostaining in 24 cases. RESULTS: ICC was successful in 33 cases (18 CB and 15 CS) and not evaluable in 5 cases (4 CB and 1 CS). The CD10-BCL6-MUM1 algorithm subclassified DLBCL as GCB (9 cases) and non-GCB (24 cases). ICC data were confirmed on histologic staining in 24 cases. CONCLUSIONS: CD10, BCL6, and MUM1 ICC staining can be performed on FNA samples. The results herein prove it is reliable both on CB and CS, and is equally effective and comparable to immunohistochemistry data.

Prevalence of Paget's disease of bone in Italy.

UNLABELLED: We examined the prevalence of PDB in Italy from radiological, scintigraphic, and biochemical surveys in two Italian towns. Prevalence rates varied from 0.7% to 2.4%, were higher in males than in females, and slightly differed between the two towns. Unlike previous studies in populations of British descent, no secular trend for a decreasing prevalence emerged. INTRODUCTION: Clinical, radiological, and necropsy data from different countries suggested pronounced geographical variations in the prevalence of Paget's disease of bone (PDB). Despite the impact of the disease on the population, there are limited data on the prevalence of PDB in Italy. MATERIALS AND METHODS: The objective of this study was to estimate the prevalence of PDB in the district of Siena (Central Italy) and Turin (Northern Italy) from radiological, biochemical, and scintigraphic surveys. We examined a sample of 1778 consecutive pelvic radiographs performed between 1999 and 2000 at the Hospital Radiology Unit in Siena and 6609 pelvic radiographs performed in 1986-1987, 1992-1993, and 1999-2002 from the Radiology Department of Molinette Hospital in Turin. In Siena, 7906 consecutive (99m)TC-MDP bone scans performed over a 4-year period (January 2000 to May 2004) were also screened for the presence of PDB, and the prevalence of elevated alkaline phosphatase (ALP) levels (>300 UI/liter) was estimated from 7449 computerized medical records over a 3-year period (January 2000 to February 2003). The finding of PDB on the pelvic radiograph and bone scan was based on standardized radiological criteria. RESULTS: At the end of the radiological surveys, 16/1778 pelvic PDB cases (8 males and 8 females) were observed in Siena and 41/6609 (27 males and 14 females) in Turin. The crude prevalence of the disease was 0.89% in Siena and 0.62% in Turin. Given that pelvic involvement is commonly described in 60-90% of PDB patients, the estimated overall prevalence of PDB ranged from 1.0% to 1.5% in Siena and from 0.7% to 1.0% in Turin. No decrease in the prevalence of PDB was evident after comparison of prevalence rates from different periods. Biochemical analyses showed 296/7449 subjects with elevated ALP levels and normal liver enzymes, 87 of whom had confirmed diagnosis of PDB. The estimated prevalence of biochemical PDB was 1.5%. The scintigraphic survey showed a PDB prevalence of 194/7906 (2.4%), which was significantly higher than the radiological and biochemical estimates. CONCLUSIONS: Our surveys suggest that PDB in Italy has an estimated prevalence of at least 1%, comparable with that observed in United States and other European countries, but lower than that described in Britain and New Zealand. No secular trend for a decreasing prevalence of PDB was observed.

Coronally advanced flap: the post-surgical position of the gingival margin is an important factor for achieving complete root coverage.

BACKGROUND: An assessment of the factors affecting the clinical outcome of root coverage procedures may be useful in clinical decision making. The aim of this study is to investigate whether the post-surgical location of gingival margin relative to the cemento-enamel junction (CEJ) can influence the recession reduction (RecRed) and complete root coverage (CRC) following coronally advanced flap procedure (CAF). METHODS: Sixty patients, aged between 22 and 57 years, 15 males and 45 females, each showing maxillary buccal recessions (> or =2 mm) identified as Miller's Class I were consecutively enrolled. All the defects were treated by CAF procedure from 2000 to 2003 by a single operator with more than 20 years of clinical experience. Age, gender, smoking habits, and type of tooth of each patient were recorded. In addition, the following clinical data were measured or computed: recession depth, width of keratinized tissue, probing depth, distance between incisal margin (IM) and CEJ, dental hypersensitivity, clinical attachment level, distance between IM and gingival margin (IMGM), distance between IM and mucogingival junction (IMMG), and the location of gingival margin relative to the CEJ following CAF procedure (GM(1)). A multiple linear regression, and a logistic linear regression analyses were performed. RESULTS: The recession depth at baseline (Rec(T0)) and the location of the gingival margin after suturing (GM(1)) are positively correlated to recession reduction. Complete root coverage appeared to be influenced by GM(1): the more coronal the level of the gingival margin after suturing (GM(1)), the greater the probability of CRC. CONCLUSION: The location of the gingival margin relative to the cemento-enamel junction following CAF procedure seems to affect CRC.

Root curvature: differences among dental morphotypes and modifications after mechanical instrumentation.

BACKGROUND: This study was designed to describe a method for measuring root curvature of extracted teeth, compare root curvature of four different dental morphotypes before mechanical instrumentation, and measure root curvature after mechanical instrumentation. METHODS: Fifty-five maxillary (14 central incisors, 13 lateral incisors, 14 cuspids, 14 premolars) extracted teeth were analyzed. Measurements were recorded on the horizontal plane at the most apical point of the cemento-enamel junction (CEJ). A 4 mm wide root portion on the buccal aspect was selected and recorded on this plane. A digital scanner traced the circle coinciding with the profile of this root portion and calculated its radius, arc, chord, and arrow (i.e., the perpendicular bisector of the chord). Since root curvature is the inverse of the radius (1/r), the radius was related to root curvature; the arc to the mesio-distal dimension of the root portion; and the reduction of the arrow to root flattening after mechanical instrumentation. The measurements were recorded four times: before treatment (baseline), after polishing, after a first root planing, and after a second root planing. The radii of each dental morphotype (upper central incisors, upper lateral incisors, upper canines, upper premolars) recorded in the first measurement were calculated and used for the statistical analyses: one-way analysis of variance (ANOVA) test and the Tukey multiple comparison method were used to study the curvature. The measurements of the radius, the arc, and the arrow at the four times were used for the statistical analyses: the two-way ANOVA test and the Tukey multiple comparison method were applied in the study of the root modifications after mechanical instrumentation. The accuracy and reliability of the method were also evaluated. RESULTS: The mean radii of the four dental morphotypes were: central incisors: 3.613 +/- 0.258 mm; lateral incisors: 2.558 +/- 0.256 mm; canines: 2.822 +/- 0.238 mm; and premolars: 2.321 +/- 0.179 mm. The statistical analyses revealed differences among central incisors, canines, lateral incisors, and premolars. There was no statistically significant difference between lateral incisors and premolars. Regarding the root modifications after mechanical treatment, the radius did not show statistically significant differences in any of the comparisons. The arc and the arrow did not show significant differences between baseline and polishing, while they did show significant differences after the second root planing. CONCLUSIONS: This study indicates that: 1) the method of measuring is accurate and reliable; 2) there are statistically significant differences among the root curvatures of different dental morphotypes; and 3) polishing did not modify the root. A vigorous root planing did not modify root curvature, but it did reduce the mesio-distal dimension and flatten the root surface slightly.

Fine needle aspiration biopsy of intraparotid spindle cell lipoma: a case report.

Intraparotid spindle cell lipoma (SCL) of the salivary gland is a rare entity. Review of the literature revealed only two previous reports describing its cytological features. We report a case of a 44-year-old man who complained for a slowly growing, asymptomatic mass in the left parotid gland that since 12 months. Fine needle aspiration biopsy (FNAB) showed a loose collections of bland-appearing spindle cells in a myxoid background admixed with capillary fragments and some mature fat cells suggesting a diagnosis of SCL. A cytological diagnosis of mesenchymal myxoid spindle cell tumor with lipomatous differentiation, possibly an intraparotideal SCL was performed. Histological examination of the mass and the positive immunostaining for CD34 and negativity for S-100, CK-cocktail, and actin confirmed the diagnosis of SCL. The diagnosis of intraparotid SCL can be made by examining cytologic material containing mature fat with bland spindle cells in a myxoid background. FNAB diagnosis on SCL also allows to rule out other primary salivary gland tumors that may be clinically and instrumentally indistinguishable and thereby permits an appropriate surgical procedure to ensue.

Intra- and inter-rater agreement of a new classification system of gingival recession defects.

PURPOSE: Several classification systems have been proposed in the literature, but none of them has been validated by means of an appropriate statistical analysis and, therefore, it is unknown whether these systems work in the same manner among different clinicians. The aim of the present study is to investigate the intra- and inter-rater agreement of a new system to classify gingival recession defects and to evaluate its agreement among different clinicians. MATERIALS AND METHODS: A new classification system was proposed and tested considering the following three factors: the amount of keratinised tissue ( <2 or ≥2 mm), the presence / absence of non carious cervical lesions and the presence/ absence of interproximal attachment loss. Kappa-statistics were performed to analyse the intra-rater and inter-rater agreement among three blinded examiners. RESULTS: A total of 120 gingival recessions were evaluated using the new classification system. The intra-rater agreement ranged from 0.74 to 0.96 for the variable keratinised tissue, from 0.67 to 0.94 for the variable non-carious cervical lesions and from 0.70 to 0.92 for the variable interproximal attachment loss. The inter-rater agreement values ranged from 0.70 to 0.85 for keratinised tissue, from 0.54 to 0.59 for non-carious cervical lesions and from 0.54 to 0.77 for the interproximal attachment loss. CONCLUSIONS: Based on the results of this study, the proposed new classification system showed moderate to substantial agreement among investigators when used in the present study population and was therefore able to diagnose the severity of gingival recession defects.

Epithelioid variant of pleomorphic liposarcoma as potential mimic of metastatic carcinoma.

We report a case of epithelioid variant of pleomorphic liposarcoma (EPL) found in the the infrapatellar fat pad of Hoffa of a 31-year old male. Histologically, the predominant population was formed by epithelioid cells with eosinophilic or clear cytoplasm admixed with rare pleomorphic lipoblasts. The immunohistochemical panel was not helpful in the diagnosis. FISH analysis using the locus-specific indicator CHOP (12q13) dual color break apart was applied to representative formalin-fixed, paraffin-embedded tissue sections. The result of FISH indicated a rearranged CHOP (DDIT3) gene and confirmed the diagnosis of EPL. The EPL should be differentiated from a metastatic carcinoma or other type of sarcoma. In these cases a clinicopathological correlation and an exhaustive sampling of the specimen for demonstration of lipogenic areas or pleomorphic lipoblasts is always necessary. FISH with demonstration of CHOP gene rearrangement is useful in providing specific ancillary information for the difficult differential diagnosis of this case.

Unusual complication of excision of pelvic endometriosis: pseudoaneurysm of the left uterine artery.

We report on a patient who had a pseudoaneurysm arising from the left uterine artery after surgical excision of deep endometriosis. The diagnosis was based on contrast-enhanced multidetector computed tomography angiography. Transfemoral selective catheterization and embolization of the left uterine artery determined a quick improvement of the symptoms.

Giant left atrial myxoma: an unusual cause of acute pulmonary edema.

We report a case of a huge left atrial myxoma with an unusual clinical presentation characterized by acute pulmonary edema. The possible pathophysiologic mechanism has been discussed.